Objective: To explore the clinical characteristics, treatment and prognosis of
TAFRO syndrome. Methods: All patients diagnosed as
Castleman disease in Peking University People's Hospital between December 2011 and April 2019 were included.Among them,6 patients were diagnosed as
TAFRO syndrome. Medical records were studied;the clinical manifestation, laboratory test, pathology, treatment and prognosis were analyzed. Recent related literatures were reviewed. Results: The average age of six
TAFRO syndrome patients (5 males)was 41.5 years(range, 27-59 years). The patients presented as acute or subacute onset, manifested as
fever,
thrombocytopenia, polyserositis including
pleural effusion and
ascites, organomegaly,
anasarca, and renal insuffciency. One patient was accompanied by hemophagocyticsyndrome, one patient was accompanied by
hypothyroidism, six patients' serum
IL-6 was elevated, four patients had received the test of serum
VEGF and results were all elevated, six patients' HIV antibody were negative,four patients had received HHV-8
DNA test and results were all negative. For pathology, threewere plasma cell type, twowere mixed type andonewashyaline vascular type. Renal biopsies were performed in 2 patients, showing that renal thrombotic microangiopathyassociated with subacute
tubulointerstitial nephritis and secondary capillary proliferative
glomerulonephritis. CHOP
chemotherapy wereused in 2 patients,
glucocorticoid was used in 1 patient, and
glucocorticoid combined with
Rituximab or
Tocilizumab were used in 3 patients. Among them, one patient died because of
disease progression after 5 years, other five patientsare still stable. Conclusion:
TAFRO syndrome is a
rare disease, early recognition and appropriate treatment may improvethe prognosis.